Management of Severe Pediatric Constipation

Overview
Constipation is an extremely common problem in the pediatric population. Most patients can be treated with mild measures (eg, dietary changes and laxatives). However, a significant number of patients have severe constipation and require more aggressive treatments, which can be described as bowel management. Most have idiopathic constipation, which is associated with a wide spectrum of severity. A small number of patients have very severe bowel dysmotility and overlap with the group of patients considered to have intestinal pseudo-obstruction. In addition to patients with idiopathic constipation, patients who have undergone surgery for anorectal malformations, as well as those with Hirschsprung disease,[1, 2] can suffer from severe constipation and incontinence. Management of patients with these surgical problems has greatly informed care of patients with idiopathic constipation. If the patient is not toilet trained by age 3 years, the clinician should offer an artificial way to keep the patient clean and socially acceptable. This regimen essentially involves teaching the family and patient how to clean the colon once a day with an enema and to manipulate the colonic motility with diet, medications, or both when necessary to ensure that the patient does not pass stool between enemas. Understanding the difference between fecal incontinence and the phenomenon known as overflow pseudoincontinence or encopresis (which is a sequela of a severe inertia of the rectosigmoid) is vital because management approaches to these 2 types of patients differ significantly. Fecal incontinence requires a bowel management regimen that involves daily enemas because such patients are unable to voluntarily empty their colons. Patients with incontinence due to severe constipation may have overflow pseudoincontinence. Once their constipation is managed, they may be able to have voluntary bowel movements. Such patients require a bowel management regimen that involves laxatives, which help them regularly and voluntarily empty their colon. Medical treatment with enemas, laxatives, and medications has traditionally been used in these patients, with varying degrees of success.[3, 4] These treatments are often used without a specific rationale in an indiscriminate manner and often reflect the lack of understanding of the difference between patients with overflow pseudoincontinence and those with real fecal incontinence. Bowel management with an organized protocol that is implemented by a thoughtful team of physicians and nurses can have a dramatic impact on a patient with fecal incontinence. Likewise, bowel management in a patient with overflow pseudoincontinence can treat the impaction, avoid constipation, and allow fecal continence. The process of bowel management in patients with fecal incontinence is one of trial and error. With the described protocol, the vast majority of patients have success and can live reasonably normally with a good quality of life. Better understanding of the causes of poor colonic motility will dramatically improve its treatment. The ideal management for these patients would be pharmacologic agents that can induce a colonic contraction with complete emptying of the colon and can then keep the colon quiescent for the next 24 hours. Most likely, histologic abnormalities remain undetected using current histologic techniques. Also, the motility studies of the colon will hopefully reach a degree of perfection that will make them more reliable and more useful from a clinical point of view. The authors believe that eventually, an understanding of genetics will play a role in the prevention of this condition. Future developments in these areas will dramatically affect the quality of life in many children. Go to Constipation and Pediatric Constipation for complete information on these topics.

Pathophysiology
Bowel motility is one of the most complex and sophisticated functions in the human body. The colon absorbs water and functions as a reservoir. Liquid waste delivered by the small bowel into the cecum becomes solid stool in the descending and sigmoid colon. The colon has a slow motility; peristalsis seems to be less active in its distal portions. Normally, the rectosigmoid remains quiet for periods of 24-48 hours. Active peristaltic waves then develop, indicating that the rectosigmoid must be emptied. This development is perceived by the individual, who then has the capacity to voluntarily retain the stool up to a point or to empty it, depending on social circumstances. To achieve fecal continence, 3 components are necessary: sensation within the rectum, good motility of the colon, and good voluntary muscle or sphincter control. Children who have anorectal malformations lack some or all of these essential components. Children who have had surgery for Hirschsprung disease may have lost some of these components because of their pelvic surgery. All patients born with anorectal malformations (except those with pure rectal atresia, which is rare) are missing the anal canal; thus, they do not experience the exquisite sensation in this area that is very important for continence. They lack the intrinsic sensation associated with stool or gas passing through the rectum. Therefore, they may soil themselves unknowingly. In a child with an anorectal malformation whose rectum has been correctly placed within the sphincter mechanism, distention of the rectum stretches the voluntary muscles that surround the rectum and gives the child proprioception, which is another vital component of continence. The sphincter mechanism in children with imperforate anus varies across a broad spectrum ranging from a mechanism similar to that observed in a child with bowel continence to a near-total absence of muscles in the perineum (muscles that allow good control and retention of stool). The postoperative problems that occur in patients who undergo operations for imperforate anus or Hirschsprung disease do not have a defined pathophysiology. The type of repair that is performed clearly contributes to future complications. In patients with anorectal malformations, the presence of a preserved rectal reservoir plays a pivotal role. Poorly developed muscles, which are often associated with a hypodeveloped sacrum or spinal and vertebral problems (eg, hemivertebrae, tethered cord, myelomeningocele, clearly contribute to the potential for fecal and urinary incontinence. Patients with anorectal malformations and Hirschsprung disease have abnormal peristaltic waves in the colon, which result in stagnant stool or an overactive colon. The child then develops constipation or overflow pseudoincontinence (encopresis). Alternatively, a very active colon may provoke a constant passing of stool, which may significantly interfere with bowel continence.

Etiology
Idiopathic constipation is an inability or difficulty in regularly and efficiently passing stool. The etiology of this condition is unknown. It is by far the most common defecation disorder and the most common colonic motility disorder in children. Idiopathic constipation affects an enormous pediatric population and represents a very common cause for surgical consultation. Idiopathic constipation is relevant not only because it affects millions of patients but also because it is extremely incapacitating in its most serious form. It can produce overflow pseudoincontinence, or encopresis (to be distinguished from true fecal incontinence). Also, the most serious type of constipation cannot be differentiated from intestinal pseudo-obstruction, a very serious motility disorder that carries a significant mortality rate. Even when the cause of this condition is unknown, the literature presents many potential causes for the disease, most of which have no solid scientific basis. Many publications discuss dietary disorders as a cause of constipation. Different types of food have either a laxative or constipating effect on the body.

Diet is important to regulate colonic motility; however, the therapeutic value of the diet is negligible in the most serious forms of constipation. Thousands of patients with mild forms of constipation are successfully managed with dietary measures alone. However, patients in whom surgery is indicated have a much more serious form of this condition that does not respond to dietary treatment.

History
Constipation after surgery
Children with fecal incontinence after imperforate anus repair can be categorized into the following 2 welldefined groups, which require individualized treatment plans: those with slow motility and those with fast motility. Children with constipation (ie, slow motility) after imperforate anus repair have usually undergone a procedure in which the rectum was preserved (eg, anoplasty, posterior sagittal anorectoplasty, sacroperineal pull-through procedure). The vast majority of patients are included in this category. Children with a tendency to have diarrhea (ie, fast motility) after imperforate anus repair have usually undergone a procedure in which the rectum and sometimes the sigmoid colonwas resected (eg, abdominoperineal pull-through procedures, endorectal resections). Occasionally, the child has lost a portion of the colon for some other reason or has a condition that produces diarrhea. The patients capacity for fecal continence (ie, the ability to hold stool voluntarily) varies according to the type of anorectal malformation with which the patient was born. High malformations are often accompanied by poor muscles, whereas low malformations are usually associated with good muscles. The degree of hypodevelopment of the sacrum and the presence of associated spinal anomalies contribute to the degree of fecal continence. For instance, a child with a high anomaly and near-normal muscles, sacrum, and spine has a good chance of achieving fecal continence, although this combination is rare. Children who have undergone surgery for Hirschsprung disease often have postoperative constipation. A small group of patients have fecal incontinence as sequelae of the original pelvic operation, which usually manifests as constipation with the inability to voluntarily empty their colon.

Idiopathic constipation
Idiopathic constipation is a self-perpetuating and self-aggravating disease. A patient that has a certain degree of constipation that is not adequately treated only partially empties the colon throughout life, leaving larger and larger amounts of stool inside the rectosigmoid, which results in greater degrees of megasigmoid. Most surgeons accept that the dilatation of a hollow viscus produces poor peristalsis. This explains why constipation means fecal retention, which produces megacolon that exacerbates the constipation. In addition, the passage of large, hard pieces of stool may produce painful anal lacerations (fissures), which result in a reluctance by the patient to have bowel movements. Consequently, if the patient was born with a certain degree of constipation and does not receive proper treatment, the constipation worsens and becomes an increasingly serious problem. The condition is mostly incurable, which means that these patients must be monitored for life. Treatments are frequently administered on a temporary basis; they are then tapered or interrupted, followed by a subsequent recurrence. This creates a great deal of frustration for patients and parents and may contribute to the well-known pattern of patients who seek a solution from many different doctors or clinics. Another controversy involves symptom onset. Many doctors believe that this problem starts during toilet training. Although symptoms become more evident at that time, the motility disorder is present at birth. Breastfed babies may not show symptoms, because of the well-known laxative effect of human breast milk. When breastfeeding is discontinued and the patient receives formula and other foods, the symptoms

become obvious. Babies who have constipation problems while breastfeeding are likely to have severe constipation that will only worsen over time. Many times, parents report that sym ptoms began when in the patients preschool years. However, specific inquiries regarding bowel movement patterns since birth often reveal that the constipation actually started very early in life. Typically, the parents remember the first fecal impaction episode most vividly and refer to that event as the initiation of symptoms. The definition of constipation is another problem. Many pediatricians believe that healthy individuals can go 2-3 days without a bowel movement throughout their lives without having any significant implications. This principle holds true for many individuals; however, when it is applied to a patient who has demonstrated idiopathic constipation, it can interfere with effective treatment. Constipation in infants is manifested by difficult and sometimes painful bowel movements, the presence of hard stool, the passing of bloodstained large pieces of stool, and periods of 2-3 days without passing stool. When these babies receive laxatives, the parents often have to increase the amount of laxatives administered to the point of producing diarrhea before the baby can pass stool. Even with liquid stool, parents describe that the babies are incapable of having bowel movements without some form of rectal stimulation. The presence of a fissure is the first sign of trouble because it produces painful bowel movements, which tend to make the patient retain stool. Holding the stool for several days produces stool retention, which favors hardening of the stool. However, the patient eventually passes a larger and harder piece of stool that reopens the fissure, creating a vicious circle. Mild forms of this condition can usually be successfully treated by a pediatrician, who may prescribe a diet with a high content of fiber, foods that act as laxatives, or both. If the diet does not correct the problem, then the pediatrician usually prescribes stool softeners, active-ingredient laxatives, or both. Laxatives are usually administered in the recommended dosages, which are successful in many patients but not in all patients; this is understandable, given the wide spectrum of severity in this condition. Fecal impaction is a stressful event characterized by retention of stool for several days, crampy abdominal pain, and, occasionally, tenesmus. A rectal examination discloses the presence of a large mass of rock-hard stool located very low in the rectum. When laxatives are prescribed to a patient with fecal impaction, the result is an exacerbation of the abdominal pain and, occasionally, vomiting. This is a consequence of increased colonic peristalsis (produced by the laxative) acting against a colonic obstruction produced by the fecal impaction. Despite the impaction, the patient may pass liquid stool, which is a phenomenon known as paradoxical diarrhea; the liquid stool passes around the solid fecal matter, but the impaction persists. Constipation is recognized and diagnosed by most practitioners upon learning that a patient has difficulty passing stool or that a patient has not passed stool in 1-3 days. Another form of constipation that is not recognized by most physicians is characterized by multiple bowel movements throughout day, consisting of very small amounts of stool. The stool is very sticky and thick and eventually becomes only a smearing or soiling of the underwear. This is also considered constipation. Soiling of the underwear without the patients awareness is an ominous sign of bad constipation. If a patient who should have achieved bowel control soils underwear day and night and does not have spontaneous bowel movements, he or she has overflow pseudoincontinence (ie, encopresis). Children with this condition behave as fecally incontinent individuals. When the constipation is adequately treated, the great majority of these pseudoincontinent children regain complete bowel control. Diagnosis is clinical. The symptoms described above are very reliable in establishing the diagnosis. In addition, if a patient presents with symptoms similar to those described above, idiopathic constipation is statistically likely. Patients with untreated Hirschsprung disease do not soil themselves; in addition, without surgical treatment, Hirschsprung disease may be fatal. Individuals who survive are frequently malnourished and have a history of enterocolitis.

Patients with idiopathic constipation do not have real enterocolitis. They occasionally experience episodes of distention and vomiting, similar to what is observed in enterocolitis. However, they actually have fecal impaction and an added picture of viral gastroenteritis that causes severe, crampy, abdominal pain and diarrhea around the impaction. Patients with Hirschsprung disease who experience actual enterocolitis become extremely toxic and lethargic and may die.

Physical Examination
Physical examination may reveal a left lower quadrant mass, which represents a sigmoid colon filled with impacted stool. Examination of the rectum is vital to determine impaction. Also, in patients who have undergone surgery, the clinician must rule out the presence of a postoperative stricture, which is particularly possible in patients who did not undergo postoperative rectal dilations. The location and caliber of the anus should be evaluated. Patients with anorectal malformations may have an anteriorly placed anus that is incorrect. Previously undiagnosed rectovestibular or rectoperineal fistulae in female patients have been reported. Congenital anal or rectal stenosis, which is very rare, may not be detected in the newborn period.

Radiography and Magnetic Resonance Imaging

The most important radiologic study for the evaluation of patients with bowel problems is plain abdominal radiography. This helps the clinician determine how much stool is present in the colon and helps confirm or refute the history of either constipation or diarrhea. A contrast enema should be performed to evaluate the patients colonic motility. Findings from this study help determine whether patients have slow motility or fast motility. If the contrast study reveals a straight colon on the left side, the rectosigmoid was resected during imperforate anus repair, and the patient has fast motility (see the first image below). If the contrast study reveals a megarectosigmoid, the rectum was preserved during the original operation, and the patient has slow motility (see the second image below).

Contrast enema in a patient in whom the rectosigmoid was resected.

Contrast enema of a patient with megasigmoid and impacted stool.

A contrast enema performed by using hydrosoluble material (never barium) is the most valuable study to confirm the diagnosis of idiopathic constipation. The dilatation of the colon extends all the way down to the level of the levator mechanism, which is recognized because it coincides with the pubococcygeal line. The lack of dilatation of the rectum below the levator mechanism (pubococcygeal line) should not be interpreted as a transition zone or nondilated aganglionic bowel. Under normal circumstances, the anal canal and the part of the rectum below the levator mechanism are collapsed by the effect of the striated muscle tone from the sphincter mechanism. The rectum above the anal canal is extremely dilated, as is the sigmoid. The contrast enema in patients with idiopathic constipation reveals different degrees of dilatation of the rectosigmoid, as is expected in a spectrum-type condition. Most interestingly, a dramatic size discrepancy is noted between a normal transverse descending colon and an extremely dilated megarectosigmoid. These changes are actually the reverse from what is observed in patients with Hirschsprung disease. The colon in a patient with Hirschsprung disease is dilated only proximal to the aganglionic segment, which remains nondilated. MRI is an excellent tool for determining whether a patient with previous repair for imperforate anus has a correctly located rectum. A specific MRI protocol that involves placement of a rubber tube in the rectum and intravenous tubing filled with oil along the buttock crease reveals whether the rectum was placed within the sphincter mechanism.

Rectal Manometry
Rectal manometry is used by many practitioners.[5] Traditionally, it is performed by placing a balloon in the rectum while measuring the pressure of the anal canal. Under normal circumstances, when the rectal balloon is inflated, the intra-anal canal pressure decreases; this is described as the anorectal reflex. Pressure that does not decrease in the anal canal is considered abnormal and is considered a sign of lack of relaxation of the internal sphincter. This is also considered diagnostic for Hirschsprung disease.

If the patients rectum has no ganglion cells, the diagnosis of Hirschsprung disease is confirmed. Alternatively, if the rectal biopsy reveals ganglion cells, the patient has achalasia of the internal sphincter. In patients with Hirschsprung disease, the treatment is well established and accepted. In cases of achalasia of the internal sphincter, the treatment proposed by many is a myectomy or internal sphincterectomy, which is a controversial procedure. The whole issue becomes more controversial when the rectal manometry is critically scrutinized. The pressure recorded in the lumen of the anal canal, supposedly generated by the internal sphincter, is actually generated both by the internal sphincter (smooth muscle) and by the striated voluntary muscle mechanism (external sphincter and levator), which surrounds the lower rectum and the anal canal around the area of the internal sphincter. To date, no publication has clarified this serious flaw in the interpretation of manometric studies. Pressure in the anal canal is not the primary result of the contraction of the voluntary sphincter mechanism. The original manometric studies in animals were performed by using muscle relaxants, which kept the voluntary muscle mechanism paralyzed. Any changes in the pressure of the anal canal, under those circumstances, could be attributed to the effect of the smooth muscle (internal sphincter). However, none of the clinical studies published have made use of muscle relaxants. In addition, the inflation of a balloon in the rectum is assumed to produce tension on the rectal walls, which triggers some mechanism that produces a decrease in pressure in the lumen of the anal canal as a final result. However, patients who are constipated have widely varying degrees of megarectosigmoid, ranging from minimal to giant. The sizes of the balloons that are used in manometric studies are never large enough to stretch giant rectosigmoids. Thus, the inflation of a regular balloon may not be enough to stretch the rectal wall in patients with megarectum, and this underinflation may produce false results.

Histologic Findings
Rectal biopsies are usually performed with the specific purpose of ruling outHirschsprung disease. The study is usually unnecessary when the clinical picture and the radiologic findings are characteristic of idiopathic constipation. Rectal biopsies are performed if the contrast enema reveals findings that suggest aganglionosis or if the patient behaves in a way that is clinically similar to a patient with Hirschsprung disease. If the patient has episodes that simulate enterocolitis and does not soil, Hirschsprung disease is suspected. If the rectal examination reveals an empty rectum and the patient is still impacted above the reach of the finger, suspect Hirschsprung disease and perform a biopsy.

Treatment of Postoperative Constipation and Diarrhea

Recognizing the different types of fecal incontinence is vital in the treatment of a patient with fecal incontinence after surgery for anorectal malformations or Hirschsprung disease. The clinician must learn how to evaluate these patients, how to recognize the specific type of fecal incontinence, and how to implement the best treatment modality. The first step is teaching the family and patient how to clean the colon with an enema. In patients with slow motility, this is a challenge. The correct enema type and amount needed to clean the colon must be determined. Keeping the colon quiet for 24 hours until the next enema is not difficult, because the patient has slow motility. In patients with fast motility, cleaning the colon with an enema is easy. The challenge in these patients is to keep the colon quiet for 24 hours until the next enema. Controlling the hypermotility requires special diet and medications. These patients must be seen regularly. The treatment process is one of trial and error, and the vast majority of patients have success with the bowel management program within one week.

The problem of overflow pseudoincontinence must be suspected in the evaluation of a patient born with a benign anorectal malformation, which is associated with a good functional result. These patients experience severe constipation that has not been properly treated and present with what the family believes is fecal incontinence. The patient must first be disimpacted. Then, over several days, bowel control must be determined. If the patient is truly incontinent and constipated, bowel management treatment is necessary. On the other hand, if the patient has bowel control, administer a determined dose of laxative on a permanent basis with an option for a sigmoid resection, which may make management easier by significantly decreasing the laxative requirements.

Constipation in patients with incontinence

Patients with constipation (ie, slow motility) are much easier to treat than those with diarrhea. The rectum normally remains quiet for 24-48 hours, after which the rectosigmoid contracts and empties during a normal episode of defecation. The rectosigmoid then remains quiet for 1-2 days. To manage constipation, daily irrigation of the colon with an enema is used to keep the colon clean. No special diets are needed. If the rectosigmoid has been preserved, as is the case with a posterior sagittal anorectoplasty (see Imperforate Anus, Surgical Perspective), the patient often experiences varying degrees of constipation and the inability to completely empty the colon in a single bowel movement. These patients require large enemas and no specific dietary restrictions or medications. The enema is administered 1 hour after eating to take advantage of the gastric-colic reflex. The child sits on the toilet for 30-45 minutes. Phosphate enemas or saline enemas are used. Patients should never receive more than 1 phosphate enema per day, because of the risk of phosphate intoxication, hypocalcemia, and hyponatremia. An enema that is correctly administered on a daily basis should result in a bowel movement followed by a period of 24 hours of complete cleanliness. If a single phosphate enema is not enough to clean the colon, the patient requires a saline-only enema. The volume of the enema is determined by trial and error. If a saline-only enema is inadequate, high colonic washings with a Foley catheter are indicated. A number 20-24 Foley catheter is lubricated and gently introduced through the anus as high as possible (see the images below). The Foley balloon occasionally must be inflated to act as a plug.

Enema administration with a tube.

Enema with inflated Foley balloon catheter.

If the child soils at any point during the following 24 hours, the bowel washout was incomplete, and a more aggressive technique is required. The volume of saline can be increased, or a second saline enema can be administered 30 minutes later. The program is individualized, and parents and children learn to examine the consistency and amount of stool obtained after the enema to determine whether it was effective.

Diarrhea in patients with incontinence

If the rectosigmoid was resected, as it is in an abdominoperineal procedure or an endorectal dissection (a procedure some surgeons use to repair imperforate anus), the patient has diarrhea (ie, fast motility) as a consequence of the loss of this section of the colon and continuously passes stool. These patients are also very sensitive to foods that provoke liquid stools. Liquid stool is problematic in children with anorectal malformations. This type of stool does not distend the rectum and leaks out without the childs knowledge. The goal of instituting bowel management in these patients is to have them form solid stool so that they are able to feel something with the voluntary muscles. Rapid transit of stool results in frequent episodes of diarrhea. Stool passes so rapidly from the cecum to the descending colon that these patients are unable to remain clean, even after administration of an enema. The bowel management program consists of teaching parents and patients a method of cleaning the colon completely every day and simultaneously determining a method to keep the colon quiet for 24 hours. To decrease diarrhea, constipating diets and medications (eg, loperamide, Lomotil) are used to slow down the colon. A small daily enema is required. Keeping the colon clean is relatively easy; keeping it quiet between enemas is difficult.

Incontinence versus overflow pseudoincontinence

The distinction between fecal continence and fecal incontinence is vital. Many patients present with severe constipation but are fecally continent. They may demonstrate signs of overflow pseudoincontinence if their colon is filled with stool and if they are impacted. Constipation management with laxatives can solve this problem. Patients who are incontinent and have constipation are very different. No matter how many laxatives are administered, they cannot empty their colon. These patients require daily enemas to completely clean the colon. Making this distinction is sometimes difficult. Occasionally, patients present with incontinence and severe constipation. Once the impaction is managed, they are continent if constipation is avoided by use of laxatives.

Trial of medical management

The authors protocol of treatment in patients with severe forms of idiopathic constipation includes a trial of medical management. If patients do not respond to this treatment, then a specific type of operation is offered. Parents often feel frustrated if the patient is offered a medical treatment that the patient has already received and failed. The authors try to convince these parents that a different protocol will be used, despite the use of the same medications (laxatives). The difference is that the dosage is adapted to the patients response. The authors assume that the patient received less laxative than required, which is often the case. In addition, the patients response is radiologically monitored. Therefore, the dosage is adjusted daily, and abdominal films are obtained every day to objectively evaluate the degree of fecal impaction.

Disimpaction
When patients come for consultation, they are usually impacted. The treatment includes the administration of 3 enemas per day (see the image below).

Administration of an enema against fecal impaction. an enema.

Administration of

The first enema of the day includes the use of a phosphate-based enema, as follows: Patients older than 12 years - 1 adult phosphate-based enema, plus 1 L of saline solution Patients aged 4-12 years - 1 pediatric phosphate-based enema, plus 1 L of saline solution Patients younger than 4 years - One half of a pediatric phosphate-based enema, plus 500 mL of saline solution The second and third enema of the day includes only the saline solution and no phosphate-based enema. The authors try to avoid the overuse of phosphate-based enemas because of the risk of phosphate absorption, which can lead to hypocalcemia. The parents give this treatment for 3 consecutive days. If they believe at some point that the patient is disimpacted, on the basis of observing the patient passing an enormous amount of stool, the patient is brought back to the clinic. Many times, when the patient passes a large amount of stool, the parents believe that the patient is disimpacted when this is not actually the case. For this reason, the authors routinely perform abdominal radiography in this setting to determine whether the colon is really empty of stool. If the patient receives 3 enemas per day for 3 days and remains impacted, he or she is then admitted to the hospital. After admission, a regimen of the same 3 enemas per day is continued, but a balanced electrolyte solution called GoLYTELY (Braintree Laboratories, Braintree, Mass) is also administered through a nasogastric tube at a rate of 25 mL/kg/h for 4 hours. Every 4 hours, the patient is evaluated. If hard stool is suspected in the abdomen, abdominal radiography is performed to ensure that the patient is disimpacted. The patient receives intravenous (IV) fluids and receives nothing by mouth during the administration of G oLYTELY. In the authors view, if the patient receives this regimen for 3 days and is still impacted, he or she should be surgically disimpacted under anesthesia. Remember not to prescribe laxatives to a patient who is fecally impacted, because it may provoke vomiting and severe abdominal pain. In addition, these symptoms cause the patient to become reluctant to take laxatives in the future. This is why the authors make a point of disimpacting first and then administering laxatives.

Postdisimpaction administration of laxatives and enemas

Once the patient has been disimpacted, the authors administer an arbitrary amount of laxative (usually a Senna derivative). The initial amount is determined on the basis of the information the parents provide regarding the previous response to laxatives. An acceptable dose is chosen, and the patient is observed for 24 hours. If the patient does not have a bowel movement within 24 hours following laxative administration, the dose was insufficient. The amount of laxative is then increased and an enema is also administered in order to remove the stool produced during the previous 24 hours. The basic rule is that the stool in these patients

with extreme constipation should never remain in the rectosigmoid longer than 24 hours because it becomes hard and is more difficult to expel in the following days. The authors continue the routine of increasing the amount of laxatives and administering an enema every night until the goal is achievednamely, the production of bowel movements and the complete emptying of the colon. Radiography is performed on the day in which the patient has a bowel movement (which is usually with diarrhea) to ensure that the bowel movement was effective, meaning that the patient completely emptied the rectosigmoid. If the patient passed stool but did not completely empty the rectosigmoid, the amount of laxatives should continue to be increased. Because idiopathic constipation has a wide spectrum of severity, the authors have encountered patients with laxative requirements much larger than expected. Occasionally, in the process of increasing the amount of laxatives, the authors find patients that vomit before reaching any positive effect. In these patients, a different medication may be tried to see if it is better tolerated. Some patients vomit all types of laxatives, feel very sick, have severe cramps, and are never able to reach the amount of laxative capable of producing a bowel movement that empties the colon. Such patients are considered intractable and, therefore, candidates for surgical intervention. However, it is usually possible to find the dosage that the patient needs to empty the colon completely, as demonstrated radiologically. Once that amount has been reached, the patient can generally be expected to stop soiling. If the patient soils at this stage, magnetic resonance imaging (MRI) of the spine and radiography of the sacrum are performed (see Radiography and Magnetic Resonance Imaging). If these results are normal, the patient may have an important psychological condition, a psychiatric condition, or both. Under these circumstances, the authors suggest the administration of a daily enema as a way to avoid impaction and to avoid soiling during the time that the patient receives adequate psychiatric help. As noted, however, the authors are usually successful in determining the amount of laxative that the patient needs, and the patient remains clean. At this point, the patient and parents have the opportunity to evaluate the quality of life that the regimen may provide. The authors explain that the treatment is most likely lifelong. In fact, as the patient ages, more laxatives may be needed. After giving the family and the patient a few days or weeks to evaluate the quality of life that they have with this treatment, the authors discuss the option of surgery with the parents. Because the origin of this condition is unknown, a rationally based treatment to cure these patients has not been found. However, an operation that provides symptomatic improvement is available and occasionally allows the patient to discontinue laxative administration and live a normal life.

Indications for Surgical Therapy

Bowel management is indicated in patients with fecal incontinence after surgical repair of imperforate anus or Hirschsprung disease. It is also used in patients who are continent after surgical repair but suffer from constipation. Surgical therapy is occasionally used.[6] The 2 procedures described for bowel management are colonic resection and access for retrograde enemas. Certain patients may be candidates for sigmoid resection. This procedure is indicated in a small subset of patients with massive dilatation of their rectosigmoid, in whom a bowel management regimen has been successful. The goal of the resection is to reduce the amount of laxatives required to empty the colon on a daily basis and to, therefore, improve the patients quality of life. Access for retrograde enemas is obtained to change the route of enema administration. At a certain age, patients desire more independence for their enema administration and want to avoid the rectal route. To this end, the authors recommend an appendicostomy placed in the umbilicus, which allows patients to administer enemas themselves. This procedure is indicated only after a successful bowel management regimen has been implemented. Other authors have used a right lower quadrant appendicostomy or a cecostomy tube for this purpose.[7]

Patients in whom resective therapy is considered to assist with bowel management must be fecally continent with a bowel management regimen. Resection of part of the colon in a patient with fecal incontinence can worsen the condition from a tendency to form solid stool to a tendency to make loose stool. Patients with incontinence and loose stool are much harder to manage than those who can form solid stool. Therefore, sigmoid resection is only used in patients who can voluntarily empty their colon, albeit with massive doses of laxatives. These patients require much smaller doses of laxatives after the surgery.

Sigmoid Resection
Sigmoid resection may be indicated in a small subset of patients who are continent but have a huge laxative requirement. In this group of patients, if a megasigmoid is revealed by using a contrast enema, a sigmoid resection can be performed to reduce the laxative requirement and improve quality of life. A transanal rectosigmoid resection can also be considered in patients with a huge rectosigmoid and an enormous laxative requirement. In a patient with a history of imperforate anuswho, by definition, lacks an original anal canal and may have poor sphinctersleaving the distal rectum in place is important because it is needed as a reservoir and to detect the presence of stool (proprioception). In a patient with severe idiopathic constipation, in whom the anal canal and sphincters are normal, a more dramatic resection can be contemplated because these patients are better able to deal with the hypermotility of the pulled-through sigmoid. Both of these operations can only be performed once a patient has been proven to have voluntary bowel movements, albeit with significant doses of laxatives. The most dilated part of the colon is resected because that part of the colon is believed to be most seriously affected. The nondilated part of the colon is assumed to have normal motility, which is why the authors use it to anastomose to the rectum. The authors have found that the patients who improve the most are the patients who have a more localized form of megarectosigmoid. Patients with more generalized forms of dilated colon do not respond as well to resective therapy. Whether a resection of a longer segment of colon in these patients should be performed is still under consideration.[8] Performing a sigmoid resection in a patient who is incontinent with constipation is a major error. [8, 9] When the sigmoid is removed, the patient becomes incontinent with loose stools, a state that is much more difficult to manage. Also, patients should not have any laxatives in their systems before a sigmoid resection is performed. Complications of a sigmoid resection include a leak at the anastomosis. This complication is the same as that which is typically faced in colon resections for other clinical situations.

Appendicostomy or Cecostomy
In patients who are fecally incontinent, a bowel management program with a daily enema is the correct treatment. The rectal route may be problematic in teenaged patients who require enemas: teenagers tend to seek independence and do not want their parents to give them enemas. In these patients, a continent appendicostomy (Malone procedure) or cecostomy for antegrade rectal washout can be performed. The operation involves connecting the appendix to the abdominal wall and fashioning a valve mechanism that allows catheterization of the appendix but avoids leakage of stool through it. Some authors insert a cecostomy tube, which requires a synthetic tubing material that enters the cecum for the same goal of performing enemas. Both procedures have been laparoscopically performed. Occasionally, if the patient has had the appendix removed, a neoappendix can be created with a cecal flap.

The colon must be completely cleaned. Regular follow-up and reassessment is necessary. Often, the volume of enema needs adjustment. Rectal examination and abdominal radiography help the surgeon assess the actual cleanliness of the colon. Complications of appendicostomy include stricture and leakage, which usually require a revision of the stoma.

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